JE Kim, et al

250 Ann Dermatol

Received January 14, 2011, Revised December 6, 2011, Accepted for publication December 7, 2011

Corresponding author: Hoon Kang, Department of Dermatology, St. Paul’s Hospital, School of Medicine, The Catholic University of Korea, 180 Wangsan-ro, Dongdaemun-gu, Seoul 130-709, Korea. Tel: 82-2- 958- 2143, Fax: 82-2-969-8999, E-mail: johnkang@catholic.ac.kr

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0) which permits unrestrictednon-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Ann Dermatol Vol. 26, No. 2, 2014 http://dx.doi.org/10.5021/ad.2014.26.2.250

CASE REPORT

Solitary Congenital Erosion in a Newborn: Report of a Solitary Congenital Self-Healing Reticulohistiocytosis

Jung Eun Kim, Byung Jik Kim, Hoon Kang

Department of Dermatology, St. Paul’s Hospital, School of Medicine, The Catholic University of Korea, Seoul, Korea

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare, cutaneous, self-limited form of Langerhans cell his-tiocytosis. Whereas multiple lesions are common, a solitary lesion is rare. A 14-day-old neonate presented with a solitary, 5-mm, oval, reddish, and eroded papule with crust on the left thigh that had existed since birth. No systemic involvement was found. Histopathology revealed dense infiltration of large histiocytes with scattered eosinophils and lymphocytes in the dermis. Immunohistochemistry showed S-100 and CD1a positivity. Two months later, the skin lesion involuted spontaneously, without evidence of recurrence and extra-cutaneous involvement. On the basis of the characteristic clinical course and typical histopathological findings, a diagnosis of solitary CSHRH was made. (Ann Dermatol 26(2) 250∼253, 2014)

-Keywords-Hashimoto-Pritzker syndrome, Histiocytosis, Langerhans-cell

INTRODUCTION

Congenital self-healing reticulohistiocytosis (CSHRH), first described in 1973 by Hashimoto and Pritzker1, is a rare Langerhans cell disorder that typically presents at birth or in the neonatal period, and involutes spontaneously with-

in weeks to a few months without the involvement of any other organs2.Although CSHRH commonly presents as multiple cutane-ous papulonodules, there have been a few reports of solitary lesions in the dermatological literature since Ber-ger et al.3 first reported on a solitary variant of CSHRH4-10. Only three such cases have been reported in the Korean dermatological literature4,5. Herein, we report on an addi-tional rare case of solitary CSHRH in a 14-day-old female neonate.

CASE REPORT

A 14-day-old female neonate was brought to our depar-tment by her mother because of a skin lesion on the left thigh that had been present since birth. She was delivered vaginally at full term, as the first child, after an uncompli-cated pregnancy. Her birth weight, length, and Apgar score were within the reference ranges. She presented with a solitary, 5-mm, oval, firm, reddish, and eroded pa-pule with crust on her left anterior thigh (Fig. 1A). The physical examination was otherwise normal, without signs of hepatosplenomegaly or lymphadenopathy. Laboratory tests, including complete blood count, urinalysis, blood glucose level, liver and kidney function, chemistry profile, chest radiograph, and newborn screening tests, were unre-markable. On hematoxylin and eosin-stained section of a skin biopsy from the lesion, focal erosion and crusting of the epidermis were observed, and the dermoepidermal junction was indistinct. Within the dermis, there was a dense dermal infiltrate of large and round histiocytic cells with abundant eosinophilic cytoplasm. The nuclei were of various shapes and sizes, from irregularly round to oval or kidney-shaped without atypia. Scattered lymphocytes, eo-sinophils, and neutrophils were also seen (Fig. 2). Most histiocytic cells showed positive staining for S-100 and CD1a, indicating that these were Langerhans cells (Fig. 3).

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Fig. 2. Histopathological findings of the lesion. (A) Focal erosion and crusting of the epidermis, indistinct dermoepidermal junction, and a dense dermal infiltrate in the up-per- and mid-dermis are seen. (B) A dense dermal infiltrate consists of large and round histiocytic cells with various nuclei in shapes and sizes, from irregularly round to oval or kidney-shaped without aty-pia (A: H&E, ×40; B: H&E, ×400).

Fig. 1. Clinical photographs of congenital self-healing reticulohistiocytosis. (A) A solitary, 5-mm, oval, firm, reddish, and eroded papule with crust on the left anterior thigh at initial presentation. (B) After 2 months, the lesion clinically resolved with hypertrophic scar.

Electron microscopy was not performed. Two months later, the skin lesion resolved spontaneously with a hypertrophic scar (Fig. 1B). Recurrence and systemic in-volvement were not observed during a follow-up period of 6 months. A diagnosis of solitary CSHRH was made on the basis of the clinical and histopathological findings.

DISCUSSION

Langerhans cell histiocytosis (LCH) is characterized by monoclonal proliferation of Langerhans cells, and the diagnosis is based on the histological criteria established

by the International Histiocyte Society in 19876. The cli-nical variants of LCH consist of Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and CSHRH6. LCH mainly affects children and is com-monly recognized as a sporadic disorder. Genetic factors and viral infections are considered to be likely pathogenic factors7.CSHRH is rare, and the incidence reported in the lite-rature varies substantially. This disease has probably been underdiagnosed because the lesions regress spontaneo-usly8,9. It is characterized by congenital or neonatal isola-ted cutaneous involvement, sparing the mucosae, and

JE Kim, et al

252 Ann Dermatol

Fig. 3. Immunohistochemically, the histiocytic cells showed a positive reaction for S-100 (A) and CD1a (B) (A: S-100, ×100; B: CD1a, ×100).

Table 1. Reported cases of solitary congenital self-healing reticulohistiocytosis in the Korean literature

Authors Sex Onset/diagnosis Site Clinical

appearanceTime to

regression IHC Electron microscopy

Chun and Song4 F Birth/2 months

Chin Papule with hemorrhagic crust

NR S-100 (+) Birbeck granules

Shin et al.5 M Birth/1 day

Left heel Papule with yellowish crust

4 weeks S-100 (+), CD1a (+)

Not done

Dorjsuren et al.12 F Birth/29 days

Left sole Papule with central crust

2 months S-100 (+), CD1a (+)

Not done

Present case F Birth/14 days

Left thigh Papule with blackish crust

8 weeks S-100 (+), CD1a (+)

Not done

IHC: immunohistochemistry, F: female, M: male, NR: not reported.

with no extracutaneous involvement7-10. The lesions can be multiple or, rarely, solitary1-11. Solitary CSHRH seems to represent about 25% of the patients among all cases of CSHRH7,8. This form usually presents with a single cuta-neous red-brown nodule that is firm and painless, and the nodule tends to progress to crusting or ulceration7,8. It can develop in any anatomical site of the body, and its mean size ranges from 5 to 20 mm7,8. Only three cases of soli-tary CSHRH have been reported in the Korean derma-tological literature (Table 1)4,5,12. In our case, the cutaneous lesion had been present since birth, with no predilection areas. The clinical appearance of all four Korean cases involved a papule with crust. Systemic involvement was not detected from the laboratory results, and the lesions spontaneously regressed. Clinically, the Korean cases of are not much different from the Western cases. The histopathological findings of solitary CSHRH are the same as multiple papulonodular forms. The findings reveal

a dense dermal infiltrate composed predominantly of large histiocytes with abundant eosinophilic cytoplasm and ro-und to oval or kidney-shaped nucleus8,9. These are accom-panied by a mixed infiltrate with variable numbers of eosi-nophils and lymphocytes8,9. The diagnosis is confirmed by a positive immunohistochemical staining for CD1a and S-100, markers of Langerhans cells8,11. Electron micro-scopy examination shows Birbeck granules and laminated dense bodies in the histiocytes; however, it is usually un-necessary and not routinely performed8,11,13. Differential diagnosis includes juvenile xanthogranuloma, hemangioma, congenital Spitz nevus, congenital fibrosa-rcoma, infantile fibrous hamartoma, infantile myofibroma, and, although not a solitary lesion, benign cephalic his-tiocytosis could also be considered. In most cases, routine histology and immunohistochemistry, such as S-100 and CD1a staining, aids in excluding those diseases8-10. No treatment is recommended, and the prognosis is exce-

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llent, with the lesions regressing spontaneously within a few weeks or months8-10. In the case of solitary CSHRH, associated visceral anomalies have never been found initially or during follow-up, unlike the classic form of CSHRH, for which hematological, hepatic, bone, and lung anomalies have been reported3-5,7-10. Recurrence and late complication have also never been reported for solitary lesions3-5,7-10,12. No guidelines for the follow-up of CSHRH have been established. Concerning solitary CSHRH, Zunino- Goutorbe et al.8 recommended regular physical examina-tion for at least 2 years initially, supplemented by simple laboratory tests and radiographs, with repetition of labo-ratory analyses perhaps exclusively at 6 months and imaging studies only if required by clinical manifesta-tions. Our patient’s lesion resolved after 2 months with-out any intervention, and there was no evidence of extra-cutaneous involvement, complication, and recurrence. Our case report is significant because it provides photographic documentation of the self-regressing nature of solitary CSHRH.In summary, because of its self-healing nature, solitary CSHRH has probably been underestimated, and physi-cians may have missed the diagnosis in the past. This rare solitary CSHRH should be included in the differential diagnosis of congenital solitary cutaneous nodules, and histopathological examination is essential to differentiate between the numerous disorders.

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